title: Shapiro Shulman syndrome: a forgotten condition.
abstract: We report on a girl with cutaneous angioma and hydrocephalus who presented the characteristics of the condition described by Shapiro and Shulman. At birth, she manifested extensive pink discoloration on her face, scalp, and back. During infancy, she developed hydrocephalus, which later stabilized. Magnetic resonance angiography revealed anomalous intracranial venous drainage, which led to development of her hydrocephalus. Various authors described such a process in the 1970s and 1980s, interpreting it as a variant of Sturge-Weber syndrome, and listing it with other neurocutaneous syndromes associated with vascular nevi, but few references to this condition appear in the recent literature. We think it exists as a syndrome, and that it is not a variant of Sturge-Weber syndrome. The cutaneous anomaly has characteristics closer to those of vascular birthmarks that fade than those of the port wine stain itself, and seems a direct consequence of abnormal venous drainage rather than a primarily capillary malformation.
authors.affiliation: Cruces Hospital, Baracaldo, Vizcaya, Spain. ppprats@gmail.com
authors.forename: José María
authors.initials: JM
authors.lastname: Prats Viñas
authors.forename: Isabel
authors.initials: I
authors.lastname: Suinaga Errasti
authors.forename: Raquel
authors.initials: R
authors.lastname: Blanco Lago
mesh_terms: D002648:Child; D004802:Eosinophilia; D005260:Female; D006801:Humans; D007859:Learning Disabilities / Q000209:etiology; D008279:Magnetic Resonance Imaging; D013585:Synovitis / Q000175:diagnosis / Q000503:physiopathology